Incontinentia Pigmenti

Incontinentia pigmenti (IP) is an unusual genodermatosis occurring almost exclusively in female patients. IP is characterized by swirling hyperpigmented skin lesions and associated with a high incidence of systemic defects. Nearly one third of patients present with neurologic complications. Consequently, neurologists and other clinicians involved in the care of these patients must be able to identify the cutaneous markers and systemic findings of IP. This article discusses the clinical presentations, laboratory findings, and pathogenesis of IP. A systematic approach to the evaluation and management of patients with IP is presented.